You may be trying to access this site from a secured browser on the server. Please enable scripts and reload this page.

Enter your Email address:

Wolters Kluwer Health may email you for journal alerts and information, but is committed
to maintaining your privacy and will not share your personal information without
your express consent. For more information, please refer to our Privacy Policy.

Quick Consult: Symptoms: Joint Pain, Myalgias, Weakness, and Rash

Author Information

Dr. Wileris an assistant professor of emergency medicine and the vice chair of clinical quality, patient safety, and process improvement at the University of Colorado Denver School of Medicine and an adjunct assistant professor of emergency medicine at the Washington University School of Medicine in St. Louis.

Diagnosis: Dermatomyositis

Juvenile dermatomyositis is a rare connective tissue disease resulting from capillary vasculopathy, but it is the most common idiopathic inflammatory myopathy of childhood (85% of cases). (Arthritis Rheum 2003;49[3]:300.)

The condition is more common in girls (1:2-5), and most are diagnosed by age 10. The exact etiology is unknown, but familial predisposition and environmental triggers, including an atypical response to infection, appear to play a role. (Arthritis Rheum 2005;53[2]:166.) Adult onset dermatomyositis peaks in middle age.

The primary presenting symptoms of dermatomyositis are muscle weakness, the characteristic heliotropic rash of the upper eyelids with periorbital edema, malar facial erythema (which does not spare the nasal labial fold like that of systemic lupus erythematosus), poikiloderma of the chest and back (hyper- and hypopigmentation with telangiectasias and skin thinning/atrophy), and Gottron's papules (red scaliness of the bilateral dorsal knuckles).

The weakness is symmetric and proximal (typically deltoid, hip flexors, and neck), which may present as frequent falls or difficulty getting off the floor (positive Gower's maneuver). The clinical course of dermatomyositis can vary in severity from mild weakness to severe muscular dystrophy. Other findings include dystrophic calcinosis (30%), cutaneous ulcerations, lipoatrophy of muscle tissue, abnormal capillaries at the nail fold (tortuous and dilated), dysphonia or dysphagia (from muscle weakness), and myalgias/polyarthritis.

Currently no serum tests definitively confirm the diagnosis because the clinical presentation and serological studies vary among individuals. (Medicine [Baltimore] 2006;85[2]:111.) Diagnosing dermatomyositis is typically made by some combination of clinical presentation and findings in muscle, on MRI (Radiographics 2000 Oct; 20 Spec No: S295), and on electromyography. MRI demonstrates areas of inflammatory changes within the muscle. Muscle enzymes and erythrocyte sedimentation rate were not useful markers of disease activity. (Rheumatology [Oxford] 2006;45[10]:1255.)

The differential diagnosis of dermatomyositis includes other causes of rash and muscle weakness but is not limited to psoriasis, myasthenia gravis, other muscular dystrophies, polymyalgia rheumatica, fibromyalgia, other connective tissue diseases, hypothyroidism, electrolyte disturbances, steroid-related myopathy, and infectious myositis.

Optimal treatment of dermatomyositis has not been defined (Rheum Dis Clin North Am 2002;28[4]:833; Mayo Clin Proc 2013;88[1]:83), but glucocorticoids appear to be helpful. Immunomodulators (azathioprine, methotrexate) may also be helpful for initial or refractory therapy. (Am J Med 1993;94[4]:379.)

Poorer prognosis appears to be related to initiation of treatment more than six months after symptom onset, severity of presenting symptoms including limb or respiratory weakness, dysphagia, pulmonary or cardiac involvement, or associated malignancy. (Clin Exp Rheumatol 1996;14[3]:263.)

The patient was restarted on Plaquenil (hydroxychloroquine) in consultation with rheumatology, and scheduled for follow-up in the rheumatology clinic.

Click and Connect!Access the links in EMN by reading this issue on our website or in our iPad app, both available onwww.EM-News.com.

Enter and submit the email address you registered with. An email with instructions to reset your password will be sent to that address.

Email:

Password Sent

Link to reset your password has been sent to specified email address.

Remember me

What does "Remember me" mean?
By checking this box, you'll stay logged in until you logout. You'll get easier access to your articles, collections,
media, and all your other content, even if you close your browser or shut down your
computer.

To protect your most sensitive data and activities (like changing your password),
we'll ask you to re-enter your password when you access these services.

What if I'm on a computer that I share with others?
If you're using a public computer or you share this computer with others, we recommend
that you uncheck the "Remember me" box.